BACKGROUND Pseudotumor cerebri (PTC) is an uncommon cause of vision loss in systemic lupus erythematosus (SLE). Disbalance between cerebral spinocellular fluid (CSF) absorption/production or cerebral veins microthrombosis/stenosis are proposed physiopathological mechanisms for PTC. Headache is the most common symptom, also, patients can present with peripheral bilateral vision loss, bilateral papilloedema, elevated intracranial pressure, normal CSF composition and normal brain imaging exams. Differential diagnosis include optic neuropathy and visual field defects. PTC must be recognized and treated quickly to preserve optic nerve. Most authors report a good response to corticosteroids in SLE patients with PTC. Still, in corticosteroid refractory cases, treatment can be done with acetazolamide, serial lumbar punctures, optic nerve sheath decompression and/or ventricular shunts. We report a PTC case on a patient in her first SLE flare, that did not occur with headache, and did not respond to corticosteroids.
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